Prevalence of Sickle Cell Disease and Other Haemoglobin Variants in Calabar, Cross River State, Nigeria
Annual Research & Review in Biology,
Background: Sickle cell disease (SCD) is the commonest genetic disorder worldwide with a global prevalence of 20-25 million. About 12-15 million affected persons are in Sub-Sahara Africa with Nigeria bearing the highest burden of people living with sickle cell disease. SCD is a disease characterized as an autosomal, recessive, heterogeneous, and a monogenetic disorder caused by an A-to-T point mutation in the β-globin gene responsible for the production of abnormal hemoglobin S (HbS), which polymerizes in the deoxygenated state and results in the sickling of erythrocytes. Haemoglobin variants are mutant forms of haemoglobin in a population usually occurring as a result of genetic changes in specific genes, or globins that causes change on alterations in the amino acid. They could affect the structure, behavior, the production rate and the stability of the specific gene. Well-known haemoglobin variants such as sick-cell anaemia are responsible for diseases and are considered haemoglobinopathies. Other variants cause no detectable pathology and are thus considered as non-pathological variants.
Aim: The study is aimed at evaluating the burden of sickle cell disease and other haemoglobin variants in Calabar, South-South Nigeria.
Methods: This is a retrospective study done at the haematology laboratory of University of Calabar Teaching Hospital, Calabar. Cellulose acetate electrophoresis at alkaline pH was used for the evaluation of haemoglobinopathies. The data were entered into Microsoft Excel 2016 spreadsheet and analysed with the IBM SPSS Version 22. Data were summarized into percentage of different phenotypes.
Results: Results of the total 3648 haemoglobin electrophoresis recorded, 1368 (37.50%) were male while the remaining 2280 (62.5%) females given a male to female ratio of 1:1.7. Five haemoglobin phenotypes were identified as HbAA, HbAS, HbAC, HbSC and HbSS. The overall average values of their prevalence were HbAA 64.78%, HbAS 32.62%, HbSS 2.14%, HbAC 0.33%, HbSC 0.14%. Thus, the prevalence of SCD (Prevalence of HbSS+HbSC) was 2.28%. The highest proportion of SCD was observed in 2011 with least in 2016 and 2017 respectively.
Conclusion: The prevalence of SCD and other haemoglobin variants in Calabar is similar to that of the national prevalence rate. There is need for continuous enlightenment and premarital counselling on the pattern of inheritance of SCD most especially with the increased burden of sickle traits in the environment has reported in this study.
- sickle cell disease
How to Cite
Lichtman MA, Kipps TJ, Seligsohn U, Kaushansky K, Paschal TJ. Disorders of haemoglobin structure: Sickle cell anaemia & related abnormalities’ in Williams Haematology, 8th edition. Landsteiner publications Chapter. 2010;48:871-914.
Hoffbrand AV, Moss PAH, Moss Petit JE. Genetic disorders of haemoglobin: In Essential Haematology, 5th edition, UK, Blackwell Publishing Ltd. 2006;6:72-93.
Akaba K, Ofem E, Bassey OB, Oluwakorede B, Riman O. Biochemical assessment of the liver in SCD in a tertiary hospital in South-South, Nigeria. Journal of Advances in Medicine and Medical Research. 2019;29(7):1-6.
Babadoko AA, Ibinaye PO, Hassan A. Autosplenectomy of sickle cell disease in Zaria, Nigeria. Oman Med. J. 2012;27(2): 121-123.
Akaba K, Inyama M, Ekwere T, Iheanacho O, Bassey E, Ushie G, Archibong H, Efiok E. Haemostatic disorders in sickle cell disease subjects in Nigeria: A review of literature. International Blood Research & Reviews. 2018;8(4):1-7.
Nwafor A, Banigo BM. A comparison of measured and predicted state. Nig. J. Appl. Sci. Environ Mangt. 2001;5(1):79-81.
Olagunju OE, Faremi FA, Olaifa O. Prevalence and burden of sickle cell disease among undergraduates of Obafemi Awolowo University, Ile-Ife. Journal of Community Medicine and Primary Health Care. 2017;29(1):74-80.
Nwogoh B, Adewoyin A, Iheanacho OE, Bazuaye GN. Prevalence of haemoglobin variants in Benin City, Nigeria. Ann Biomed Sci. 2012;11(2):61-64.
Adu EM, Isibor CN, Ezie E. Prevalence of haemoglobin variants among the Ika ethnic nationality of Delta state. Int J Med Biomed Res. 2014;3(2):63-67.
Udomah FP, Isaac IZ, Aliyu N, Erhabor O, Ahmed MH, et al. Haemoglobin electrophoretic patterns, ABO and Rhesus D blood groups distribution among antenatal women in Sokoto, Nigeria. Obstet Gynecol Cases Rev. 2015;2:34- 38.
Odunvbun ME, Okolo AA, Rehimy CM. Newborn screening for sickle disease in a Nigerian hospital. Public Health. 2008; 122:1111-1116.
Baba PI, Daniel Y, Lawson JO, Dada J, Matthews CE, Sukhleen M, Obaro SK. Sickle cell disease screening in Northern Nigeria: The co-existence of B-thalassemia inheritance. Paediatrics & Therapeutics. 2015;5(3):1-4.
Akhigbe RE, Ige SF, Afolabi AO, Azeez OM, Adegunlola GJ, Bamidele JO. Prevalence of haemoglobin variants, ABO and Rhesus Blood Groups in Ladoke Akintola University of Technology, Ogbomoso, Nigeria. Trends Med. Res. 2009;4:24-29.
Akinboro A, Komolafe EK, Musibau AAA retrospective study on fourteen-year haemoglobin genotype variants recorded at five government hospitals in Akure, Ondo State, Southwestern Nigeria. The Egyptian Journal of Medical Human Genetics. 2016;17:377-381.
Bakare AA, Azeez MA, Agbolade JO, Gene frequencies of ABO and rhesus blood groups and haemoglobin variants in Ogbomoso, South-West, Nigeria. Global J. Med. Sci. 2004;3:17-22.
Akinyanju OO. A profile of sickle cell disease in Nigeria. Annals of the New York Academy of Sciences. 1989;565(1 sickle cell D):126-136.
Nnaji GA, Ezeagwuna DA, Nnaji IJF, Osakwe JO, Nwigwe AC, Onwurah OW. Prevalence and pattern of sickle cell disease in premarital couples in Southeastern Nigeria. Nig. J. Clin Prac. 2013;16(3):1-6.
David AN, Jinadu MY, Wapmuk AE, Gbajabiamila TA, Okwuzu O, Herbertson EC, Ezechi OC. Prevalence and impact of sickle cell trait on the clinical and laboratory parameter of HIV infected children in Lagos, Nigeria. 2018;31:113-120.
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